The UMD-FBN1 mutations database
Record ID: 246

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.IVS39-1G>C (c.4943-1G>C)HeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
GATAspspl-1Spl.G->CTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #24 Ca2+ binding

Mutation impact


At the mRNA levelOn restriction map
Skipping of exon 40, in frameNew restriction site(s): none
Lost restriction site(s): none

Impact on splicing
Splice site type Wild type sequence CV Mutant type sequence CV Variation (%)
Acceptor
tttggattatagAT
79.8 _
tttggattatacAT
50.8 _ *
-36.3 %

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA03STA F0034 I01ProbandNANA?U.S.A

Phenotypic groupDisease
NAClassical MFS

Clinical data


Symptom
no clinical data

Reference


Reference IDPubMed IDReference
5410464652
Liu WO, Oefner PJ, Qian C, Odom RS, Francke U. "Denaturing HPLC-identified novel FBN1 mutations, polymorphisms, and sequence variants in Marfan syndrome and related connective tissue disorders". Genet Test 1997-98;1(4):237-42.